This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Neurochemical Correlates of Pathology in Spinocerebellar Ataxias (in collaboration with Dr.s Harry Orr and Brent Clark, Lab Medicine and Pathology and Huda Y. Zoghbi, Baylor College of Medicine, supported by R21 NS060253 and Bob Allison Ataxia Research Center, PI Oz): In this project our goal is to validate the MRS markers of neurodegeneration by comparison to histology findings in the same brains. For this purpose we utilize multiple mouse lines that carry the SCA1 mutation, including a treatment model, in order to determine if high field MRS is sensitive to early, pre-symptomatic neurochemical alterations, as well as to the reversal of such changes with treatment.